Perivascular epithelioid cell tumor (PEComa) with perirenal manifestation.

We present the first case report documenting an 8-month post-surgical follow-up of an exceedingly rare perivascular epithelioid cell tumor (PEComa) with a perirenal location. Furthermore, prognostic parameters discussed in the literature are summarized and concise information regarding imaging characteristics on magnetic resonance imaging is provided. In conclusion, our report points toward PEComa as a family of very rare mesenchymal neoplasms that should be included into deliberations concerning the differential diagnosis of perirenal mass lesions.